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Blood, Vol. 95 No. 2 (January 15), 2000:
pp. 398-403
Molecular analysis and clinical outcome of adult APL patients with
the type V PML-RAR isoform: results from Intergroup protocol 0129
James L. Slack,
Cheryl L. Willman,
Janet W. Andersen,
Yun-Ping Li,
David S. Viswanatha,
Clara D. Bloomfield,
Martin S. Tallman, and
Robert E. Gallagher
From the Department of Medicine, Roswell Park Cancer Institute,
Buffalo, NY; Department of Pathology, University of New Mexico Cancer
Center, Albuquerque, NM; Department of Biostatistics,
Harvard School of Public Health, Boston, MA; Departments of
Oncology and Medicine, Montefiore Medical Center and Albert Einstein
Cancer Center, Bronx, NY; Department of Medicine, The Ohio State
University Comprehensive Cancer Center, Columbus, OH; and Department of
Medicine, Northwestern University Medical School, Chicago, IL.
The type V (for variable) promyelocytic leukemia
retinoic acid receptor (PML-RAR) transcript, found in approximately
8% of adult patients with acute promyelocytic leukemia (APL), is
defined molecularly by truncation of PML exon 6 and frequent insertion of genetic material from RAR intron 2. To more fully characterize the molecular features of PML-RAR V-type transcripts and to
determine whether V-form APL patients have a distinct clinical
presentation or prognosis, we analyzed 18 adult V-form APL patients
enrolled on Intergroup protocol 0129 (INT-0129). Truncations in PML
exon 6 ranged from 8 to 146 nucleotides, and 3 to 127 extra nucleotides (1 to 42 extra amino acids) were inserted at the PML exon 6/RAR exon
3 junction in 13 cases. No distinguishing morphologic, cytogenetic, or
immunophenotypic features of V-form blasts were identified. A total of
5 of 7 patients induced with ATRA and 8 of 11 patients who received
chemotherapy for induction achieved complete remission (CR). Six
patients have relapsed, 4 after chemotherapy induction and 2 after
ATRA. Nine patients (50%) are alive, 6 in continuous CR, 2 after
salvage therapy for relapsed or refractory disease, and 1 after
alternative treatment following early removal from protocol. Although
the failure rate for V-form APL patients was high (61%), the low power
of the current study to detect clinically significant differences
precludes a meaningful comparison of clinical outcomes between the 18 V-form cases and non-V-form adult APL patients enrolled on INT-0129.
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